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Expression of nerve‐regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies
Author(s) -
Sedehizade Fariba,
Klocke Rainer,
Jockusch Harald
Publication year - 1997
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199702)20:2<186::aid-mus8>3.0.co;2-8
Subject(s) - myogenin , muscular dystrophy , endocrinology , denervation , neuromuscular junction , duchenne muscular dystrophy , medicine , biology , myod , skeletal muscle , myogenesis , neuroscience
The expression of the genes for the α‐subunit of AChR (AChRα), for the myogenic factors myogenin and MyoD, for the calcium‐binding protein parvalbumin (PV), and for the muscular chloride channel CIC‐1 was studied in the three mouse spinal muscular atrophies (SMAs). These were the mutants “wobbler” (WR), “muscle deficient” (MDF) and “progressive motor neuronopathy” (PMN). Murine myopathies “muscular dystrophy with myositis” (MDM) and “X‐linked muscular dystrophy” (MDX) were used as controls. AChRα and myogenin mRNA levels were strongly elevated in muscles affected by SMAs (reflecting denervation), whereas only myogenin mRNA was moderately elevated in MDX and MDM muscles, probably due to fiber regeneration. As in denervated muscle, CIC‐1 and PV mRNA levels were lowered in SMAs. No changes were seen in muscles of up to 222‐day‐old symptomless ciliary neurotrophic factor (CNTF) knockout mice. The patterns of gene expression were characteristic for the type of muscle disease, indicating their possible usefulness for clinical diagnosis. © 1997 John Wiley & Sons, Inc. Muscle Nerve , 20, 186–194, 1997.